Objective: To review and analyze the clinical and pathological data of children with
autoimmune hepatitis (AIH). Methods: Medical records of 46 patients hospitalized in Pediatric
Liver Diseases Treatment and Research Center, Fifth Medical Center, General Hospital of People's Liberation Army(PLA) from April 2012 to April 2018 were extracted. Medical data included type of AIH, clinical manifestations, biochemical parameters, liver biopsy results, and outcomes of treatment were analyzed retrospectively. Among 46 children, 19 were males and 27 were females. The age of onset was 10.1(1.4-18.0) years old. Chi-Square test, Rank sum test or t test were used for inter-group comparison. Results: There were 32 (70%)AIH-I cases and 14 (30%)AIH-Ⅱ cases (χ(2)=12.565, P=0.000). Among the 46 patients, there were 5 modes of onest: 17 cases (37%) had acute viral
hepatitis-like presentation, 2 cases (4%) had
fulminant hepatic failure, 9 cases (20%) had insidious onset, 5 cases (11%) showed
cirrhosis and
portal hypertension, and 13 cases (28%) were incidentally found to be due to elevated hepatic
aminotransferases. Comorbidities including primary sclerotic
cholangitis (n=3),
primary biliary cholangitis (n=1),
systemic lupus erythematosus (n=1) and
inflammatory bowel disease (n=2), were all seen in AIH-Ⅰ cases. The elevated biochemical parameters of these patients were as follows:
alanine aminotransferase (n=46),
aspartate transminase (n=46), total
bilirubin (n=35) γ-glutamyl transpeptadase (n=39), γ-
globulin (n=32) and
IgG (n=33). The γ-
globulin and
IgG levels were significantly higher in AIH-Ⅰ patients than those with AIH-Ⅱ((32±9)% vs. (23±8)%, t=3.217, P=0.002,(27±10) vs. (18±8)g/L, t=3.193, P=0.003, respectively). Thirty-nine patients received liver biopsy, among whom 22 (56%) with
inflammation grade (G)≥3, 26(67%) with
fibrosis stage (S) ≥3, and 7 with
hepatic cirrhosis (S4) according to pathological analysis. Typical histopathological changes of AIH included: 36 cases of interfacial
hepatitis (92%), 23 cases of lymphocyte/plasma cell infiltration (59%), 3 cases of rosette (8%). Forty patients received
prednisolone monotherapy or combined with
azathioprine after diagnosis. Complete remission was seen in 29 (72%) patients, partial remission in 10 (25%) patients and no response in 1 (3%) patient. Among complete remission patients, 15 (52%) had relapse in the process of
prednisolone reduction. Repeated liver biopsy performed in 8 patients
after treatment showed that hepatic
inflammation and
fibrosis were both improved in 6 patients, only
inflammation was alleviated without
fibrosis improvement in 1 patient, and neither
inflammation nor
fibrosis was improved in 1 case. The length of follow-up was 3.3 (0.3-10.5) years, and none of the 39
prednisolone-responded cases discontinued treatment successfully. Adverse effect of long-term
prednisolone therapy included bilateral
cataract (n=6),
spinal fracture accompanied with delayed bone age development (n=1). Conclusions: AIH-Ⅰ is more common than AIH-Ⅱ in children, with diverse clinical characteristics. Most cases have progressive liver
inflammation and
fibrosis when diagnosed.
Prednisolone monotherapy or combined with
azathioprine could achieve both biochemical and pathological improvement, but relapse is inevitable during
drug tapering, hence long-term treatment is essential.